1/7. Acute renal infarction. Clinical characteristics of 17 patients.We analyzed the medical records of patients with an established diagnosis of acute renal infarction to identify predictive parameters of this rare disease. Seventeen patients (8 male) who were admitted to our emergency department between May 1994 and January 1998 were diagnosed by contrast-enhanced computed tomography (CT) as having acute renal infarction (0.007% of all patients). We screened the records of the 17 patients for a history with increased risk for thromboembolism, clinical symptoms, and urine and blood laboratory results known to be associated with acute renal infarction. A history with increased risk for thromboembolism with 1 or more risk factors was found in 14 of 17 patients (82%); risk factors were atrial fibrillation (n = 11), previous embolism (n = 6), mitral stenosis (n = 6), hypertension (n = 9), and ischemic cardiac disease (n = 7). All patients reported persisting pain predominantly from the flank (n = 11), abdomen (n = 4), and lower back (n = 2). On admission, elevated serum lactate dehydrogenase was found in 16 (94%) patients, and hematuria was found in 12 (71%) of 17 patients. After 24 hours all patients showed an elevated serum lactate dehydrogenase, and 14 (82%) had a positive test for hematuria. Our findings suggest that in all patients presenting with the triad--high risk of a thromboembolic event, persisting flank/abdominal/lower back pain, elevated serum levels of lactate dehydrogenase and/or hematuria within 24 hours after pain onset--contrast-enhanced CT should be performed as soon as possible to rule out or to prove acute renal infarction.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/7. Thrombotic thrombocytopenic purpura presenting as bilateral flank pain and hematuria: a case report.Thrombotic thrombocytopenic purpura (TTP) is a rare disease whose incidence is now increasing. We present a case of a 37-year-old man who presented with bilateral flank pain and hematuria, subsequently diagnosed with TTP. Thrombotic thrombocytopenic purpura has classically been characterized by the pentad of fever, microangiopathic hemolytic anemia, neurologic symptoms, renal dysfunction, and thrombocytopenia. The pathogenesis of the disease has been a mystery until recently. We review the current literature regarding the pathophysiology and management of this disorder. Our discussion focuses on the importance of understanding this disease while considering the differential diagnosis of a patient presenting with anemia and thrombocytopenia because the common pitfall of rapidly administering platelets to a patient with TTP may lead to a disastrous outcome.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/7. nephrolithiasis and hematuria--sometimes a stony road to diagnosis.We report a case of a young man with a history of kidney stones. Occurrence of gross hematuria several months after the extracorporeal shock wave, lithotripsy (ESWL) treatment lead to hospitalization. By ultrasound and abdominal CT scan, the urologist could exclude post-renal causes of the gross hematuria and acute renal failure. After transfer to a department of nephrology hemodialysis was started, an immediate kidney biopsy was performed and prednisolone was administered on the same day. The kidney biopsy revealed an anti-glomerular basement membrane (GBM) disease. The renal function did not recover and the patient remained on hemodialysis. In the literature it has been hypothesized that ESWL-treated patients are prone to develop anti-GBM disease by liberation of glomerular basement antigen through the ESWL high energy shock waves. An additional hypothesis considering the higher susceptibility for anti-GBM disease among certain HLA-tissue types is discussed with regard to our case. Unfortunately, the prolonged track to diagnosis and delayed immunosuppressive treatment could not prevent poor clinical outcome. Although anti-GBM disease is a rather rare disease, it should be included as a differential diagnosis for hematuria--especially months after ESWL treatment. Otherwise early diagnosis may be missed and as in our patient immunosuppressive treatment will remain unsuccessful to recover renal function.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/7. Primary amyloidosis of the urinary bladder.amyloidosis is a systemic disease that usually occurs in the gastrointestinal tract or in muscular or adipose tissue. Primary amyloidosis of the urinary bladder is a rare disease that can mimic bladder cancer on cystoscopic examination as well as in its clinical presentation of painless gross hematuria. This report describes a 49-year-old male with repeated painless gross hematuria, who underwent transurethral resection of a suspected bladder tumor. Pathologic examination revealed papillary urothelial hyperplasia with vascular ectasia and no signs of malignancy. Massive gross hematuria occurred 2.5 years later. cystoscopy showed multiple papillary lesions with yellowish-brown submucosal plaques on the posterior bladder wall. A second transurethral tumor resection was performed and histologic examination revealed plasma cell infiltration and eosinophilic amorphous deposits in the subepithelial stroma and vascular wall. The deposits were positive for congo red and apple-green birefringence under polarized light examination but negative for Masson's trichrome stain, indicating that they were not fibrotic in nature. Hence, the diagnosis of amyloidosis of the urinary bladder was confirmed. Screening for amyloidosis was negative in other organ systems and the patient has remained disease-free up to the last follow-up 4 years after the second transurethral resection. amyloidosis should be considered in the differential diagnosis of patients with recurrent hematuria who have symptoms characteristic of bladder cancer but negative pathologic study for malignancy. Correct diagnosis relies on clinical alertness and the use of a special staining technique during pathologic examination.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/7. Bladder carcinoma and hiv infection during the highly active antiretroviral therapy era: A rare, but intriguing association. Two case reports and literature review.Two very rare case reports of bladder transitional cell carcinoma associated with hiv infection in patients treated with combined antiretroviral therapy are described, and discussed on the ground of the most relevant and updated literature resources. Only 13 cases of vesical carcinoma have been reported to date in the setting of hiv infection, but only 3 anecdotal single reports (the last in the y 2001) described clinical, therapeutic, and outcome issues of this rare disease association in some detail. In our patients, micro- or macro-haematuria was the clue for in-depth diagnosis and prompt treatment, which was limited to multiple local interventions in 1 case, but finally required a radical cystectomy in the second patient. No relationship was found with the very favourable underlying hiv-related virological and immunological status, and the present 8-12-month follow-up did not show disease relapses.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/7. Renal cell carcinoma in pregnancy.A rare case of renal cell carcinoma occurring during pregnancy is described. The modalities used to make the diagnosis and the antepartum management of the patient are discussed. The case reported herein demonstrates the major diagnostic challenge of the young pregnant patient with a rare disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/7. Rare case of the utero-vesical fistula caused by intrauterine contraceptive device.The vesico-uterine fistula is a very rare disease. There have only been 150-200 causes. We are reporting on a case in which the chronic fistula was caused by an IUD having been placed 4 years ago and it "wandered" through the bladder. A 30 year-old patient in 1992 and IUD was inserted. She had gynecological controls twice, in 1993 last time. She has problem of urination very often. A cyclical bladder bleeding drew the attention to the disease. In ambulanterely performed cystoscopy we found an IUD perforating towards the interior of bladder in the borderline of its bottom and back wall and was situated in the bladder with its 3/4. We have removed it with forceps. After six weeks of expectation and strict observation did we want to manage the fistula after having consolidated the symptoms of the inflamed surroundings. During the operation we have noticed a wallment size mass of scar between the uterus and the bladder expanding to the height of the orifice of the uterus. The scarily fixed bladder has been separated from the cervix and the scarry wall of the fistula has been cut out. We have brained the cervix towards the vagina and then we've sutured the cervix and the bladder with Dexon 'O' treat, as well. We have interposed a surgical net between the cervix and the bladder followed by blood-clotting and peritonisation. We should take the follows into consideration: careful separation, fine operative technique, and strong well absorbing thread as well as trying to keep the organ. In our opinion the bioplast--interpositum used on our case maker the efficiency of the operation higher.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |